Treatment of Ebstein's anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias. Treatments may include:
If you have no signs or symptoms or abnormal heart rhythms, your doctor may recommend only careful monitoring of your heart condition with regular checkups. Checkups typically include a physical exam, electrocardiogram, chest X-ray, echocardiogram and, if necessary, a Holter monitor test. Your doctor may also ask you to undergo an exercise test, such as walking on a treadmill. This test checks your heart's response to physical activity.
If you have heart rhythm disturbances, medications may help control heart rate and maintain normal heart rhythm.
Your doctor may also prescribe medications for signs and symptoms of heart failure, if you need them. These may include drugs that prevent water retention (diuretics) and other medications.
Surgical heart repair
Your doctor may recommend surgical repair when your signs and symptoms are affecting your quality of life or when your heart begins to enlarge and overall heart function begins to decrease. Having surgery at a younger age may lead to better outcomes. Because Ebstein's anomaly is rare, choose a surgeon who's familiar with the defect and who has experience performing procedures to correct it. Different types of procedures can be used to surgically treat Ebstein's anomaly and associated defects, including:
Tricuspid valve repair. This involves reducing the size of the valve opening and allowing the existing valve leaflets to come together to work properly. This procedure is usually done when there's enough valve tissue to allow for repair.
A newer technique for the repair of Ebstein's anomaly, called the cone procedure, uses the existing leaflets to form a cone that works like a valve.
- Tricuspid valve replacement. If the existing valve can't be repaired, it may be replaced by removing the deformed valve and inserting either a mechanical valve or a specially treated biological tissue (bioprosthetic) valve. If a mechanical valve is used, you'll need to take a blood-thinning medication for the rest of your life.
- Closure of the atrial septal defect (ASD). If a hole is present between the two upper champers of the heart (ASD), your surgeon can close the hole during surgery to repair or replace the defective valve.
- Maze procedure. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It's used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias). To do the procedure, your surgeon makes a series of incisions in the right atrium with a scalpel or a device that destroys tissue by freezing it. When these incisions heal, the scars create a barrier of tissue in your heart that causes it to beat at a normal rate. The Maze procedure is an open-heart surgery.
Radiofrequency catheter ablation
Radiofrequency catheter ablation is another procedure that can help treat a fast heartbeat or WPW syndrome. In this procedure, one or more catheters are threaded through your blood vessels to your inner heart, where the electrical impulses that cause your heart to beat are generated. Electrodes at the catheter tips are heated with radiofrequency energy. This destroys (ablates) a small spot of heart tissue and creates an electrical block along the pathway that's causing your arrhythmia. Usually, this stops your arrhythmia. However, this procedure may be less effective for treating people with Ebstein's anomaly than for those with other heart problems. Repeat procedures may be necessary.
If you have severe Ebstein's anomaly and poor heart function, a heart transplant may be necessary.
Sept. 17, 2014
- Attenhofer JCH, et al. Ebstein's anomaly. Circulation. 2007;115:277.
- Fuster V, ed., et al. Hurst's The Heart. 13th ed. New York, N.Y.: The McGraw-Hill Companies; 2011. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=5. Accessed March 12, 2013.
- Crawford MH, ed., et al. Current Diagnosis & Treatment: Cardiology. 3rd ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=3649722. Accessed March 12, 2013.
- Silversides CK, et al. Canadian cardiovascular society 2009 consensus conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Canadian Journal of Cardiology. 2010;26;e80.
- Zhao W, et al. Pregnancy outcomes in women with Ebstein's anomaly. Archives of Gynecology and Oncology. 2012;286:881.
- Dearani JA, et al. Surgical advances in the treatment of adults with congenital heart disease. Current Opinion in Pediatrics. 2009;21:565.
- Attenhofer Jost CH, et al. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly. Journal of the American College of Cardiology. 2012;59;2101.
- Vogel M, et al. Ebstein's malformation of the tricuspid valve: Short-term outcomes of the "Cone Procedure" versus conventional surgery. Congenital Heart Disease. 2012;7:50.
- U.S. News and World Report Best Hospitals: Cardiology & Heart Surgery. U.S. News and World Report. http://health.usnews.com/best-hospitals/rankings/cardiology-and-heart-surgery. Accessed March 13, 2013.
- Grogan M (expert opinion). Mayo Clinic, Rochester, Minn. March 17, 2013.
- Dearani JA, et al. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. The Annals of Thoracic Surgery. 2013;96:202.
- Cook AJ. Decision Support System. Mayo Clinic, Rochester, Minn. April 3, 2014.
- Dearani JA (expert opinion). Mayo Clinic, Rochester, Minn. Aug. 12, 2014.
- Anderson HN, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenital Heart Disease. 2014;9:266.
- Boston US, et al. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience. The Annals of Thoracic Surgery. 2006;81:690.
- Brown ML, et al. The outcomes of operations for 539 patients with Ebstein anomaly. The Journal of Thoracic and Cardiovascular Surgery. 2008;135:1120.
- Brown ML, et al. Effect of operation for Ebstein anomaly on left ventricular function. The American Journal of Cardiology. 2008;102:1724.
- Raju V, et al. Right ventricular unloading for heart failure related to Ebstein malformation. The Annals of Thoracic Surgery. 2014;98:167.